Unusual Presentation of Fibrosarcoma in a Child.

Malignant soft tissue tumours are rare in infants. Specific radiologic features help differentiate benign from malignant musculoskeletal entities. We report a case of malignant soft tissue tumour with initial radiologic evaluation suggesting a benign entity, thus delaying diagnosis. The case was treated as a case of cystic hygroma, suggested by ultrasound and MRI with good initial response to bleomycin injection. Later, symptoms recurred with poor response to repeat therapy which led to review the diagnosis. Angiography, arterial embolisation, surgical excision, and finally histopathology showed that a cystic infantile fibrosarcoma was initially misinterpreted as cystic hygroma. A comprehensive literature review and discussion on infantile fibrosarcoma; and its mimics are provided.

Successful treatment of hydatid cyst of lesser sac with PAIR therapy.

Hydatid disease has varied presentations and may involve any part of the body with hydatid disease of the liver being the most common site. Hydatid disease of lesser sac is a rare entity. Different treatment options of hydatid liver disease are available which include surgical, medical and radiological management. Surgical management has its limitations in terms of cost, morbidity, mortality, rate of recurrence and patient fitness to undergo surgery. Medical management alone carries a low chance of cure. Radiological management with PAIR therapy (percutaneous aspiration, injection and re-aspiration) of hydatid liver disease has been well described in literature. However, hydatid disease of lesser sac in itself is a rare entity and its treatment with PAIR procedure has not been described before. We describe a case of hydatid disease of lesser sac treated with the PAIR procedure.

Congenital heart disease screening: which referral factors are most important?

OBJECTIVE: To identify the referral factors for fetal echocardiography which are associated with congenital cardiac defects in the fetus. STUDY DESIGN: Cross-sectional descriptive study. PLACE AND DURATION OF STUDY: Radiology Department, CMH, Rawalpindi, from January 2007 to November 2010. METHODOLOGY: All patients referred for fetal echocardiography with one or more risk factors for the development of congenital heart disease, and those patients with incidental discovery of congenital heart disease on antenatal ultrasound were evaluated. Patients with no risk factors who were found to have normal fetal echocardiography were excluded from the study. Univariate logistic regression analysis was carried out for each variable. The variables with statistical significance of less than 0.05 were subjected to multivariate logistic regression. Fetal echocardiographic diagnosis was taken as the dependent variable and all other variables were the independent variables. RESULTS: Two hundred and sixty four patients were evaluated by fetal echocardiography for congenital heart disease. The statistically significant factor was detection of congenital heart disease on routine ultrasound examination. CONCLUSION: A routine obstetric scan should include evaluation of the heart with four-chamber and base-of-heart views to exclude cardiac anomalies. A cardiac anomaly picked up on routine ultrasound scan is the most important indication for referral for fetal echocardiography. Fetal arrhythmias and echogenic focus in the left ventricle do not have a significant association with structural cardiac malformation.

Blood infection with Enterobacter aerogenes--an unusual cause of portal vein gas.

Portal vein gas was once thought of as an invariably fatal condition. Now, with the availability of better equipment and expertise, the condition is more frequently diagnosed. A case of fever with rigors is presented and on ultrasound and CT examination was found to have portal venous gas which resolved with adequate antibiotic treatment. Blood culture revealed growth of gram negative bacillus; Enterobacter aerogenes. Patient was investigated further for portal vein gas, and although no other cause for the development of portal vein gas was found, she was treated with antibiotics and showed an immediate response. The aim of this case report is to highlight the benign causes of portal vein gas as well as to discuss the causes which warrant immediate surgery. Portal vein gas may herald a more ominous condition, which if intercepted in its course may result in complete cure.

Multiple breast masses in a case of multiple myeloma.

Myeloma of the breast is a rare entity with only a few reported cases in the literature. It is usually secondary to adjacent bone disease with only a few instances of primary involvement of the breast. We present a rare case of plasmacytoma of left humerus that presented with multiple breast masses and skin nodules. Histopathology of the breast and skin nodules showed plasma cells consistent with the diagnosis of multiple myeloma.

Pre-natal diagnosis of caudal regression syndrome.

Caudal regression syndrome is a rare syndrome characterized by absence of the lower spine to a varying degree. It has particularly high incidence in patients with Insulin Dependent Diabetes Mellitus (IDDM). We report a case of caudal regression syndrome in a newly diagnosed case of IDDM at 30 weeks of gestation.

Vein of Galen aneurysm: antenatal diagnosis: a case report.

Aneurysm of vein of Galen is a rare congenital malformation with reported incidence of less than 1% of cerebral vascular malformations. Most cases present in neonatal life with congestive cardiac failure. We report a case of aneurysm of vein of Galen diagnosed in intrauterine life at 36 weeks of gestation. The importance of third trimester Doppler ultrasound is highlighted.

Achalasia cardia and alacrima in an infant.

Achalasia cardia is a very rare entity in children and may go undiagnosed for many months if not suspected. We report a case of a 4 months old child who presented with regurgitation of milk and solids and failure to thrive. She was initially treated as gastro-oesophageal reflux. There was a family history of achalasia cardia in the kins with resultant death in one and grossly delayed milestones in the other. Along with achalasia cardia, our patient had alacrima, which brought attention to the associations of achalasia with 'double A', 'triple A' and the '4 A' syndromes.